Clinical bottom line
Hereditary hemorrhagic telangiectasia (HHT) presents unique anesthetic challenges due to fragile vascular malformations and bleeding risks. Current evidence highlights the importance of individualized perioperative planning, careful airway management, and vigilant hemodynamic monitoring to minimize hemorrhagic complications. While data remain limited, emerging case reports and procedural outcome studies suggest that multidisciplinary coordination and tailored anesthetic protocols can improve safety in HHT patients undergoing surgery.
What the evidence shows
HHT is an autosomal dominant disorder characterized by mucocutaneous telangiectasias and arteriovenous malformations (AVMs) affecting multiple organs, including the nasal mucosa, lungs, liver, and brain (Hereditary Hemorrhagic Telangiectasia, 2021). The hallmark clinical feature is recurrent epistaxis, often severe, due to fragile nasal telangiectasias (Tunkel et al., 2020). These vascular abnormalities increase perioperative bleeding risk and complicate airway management.
The 2020 clinical practice guidelines on epistaxis emphasize the need for preoperative evaluation of bleeding severity and nasal mucosa status in HHT patients to guide anesthetic planning (Tunkel et al., 2020; Tunkel et al., 2020). Airway instrumentation should be performed with extreme caution to avoid mucosal trauma and bleeding. Regional anesthesia may be preferable when feasible to reduce airway manipulation, but the presence of AVMs in the spinal or epidural space must be ruled out.
A recent case report detailed an anesthetic protocol for enteroscopy in an HHT patient with gastrointestinal angiodysplastic lesions, highlighting the importance of maintaining stable hemodynamics and avoiding hypertension spikes that could precipitate bleeding (Yang et al., 2025). The authors used gentle induction agents, careful airway instrumentation, and invasive monitoring to promptly detect bleeding or hemodynamic instability.
Electrophysiologic procedures in HHT patients have been studied in a cohort analysis, revealing that procedural risks vary by type and location of vascular lesions (Ezeh et al., 2026). The study underscored the need for individualized risk stratification and multidisciplinary input, including cardiology and hematology, to optimize perioperative outcomes.
Overall, the evidence supports a cautious, multidisciplinary approach integrating preoperative bleeding risk assessment, tailored anesthetic techniques, and vigilant intraoperative monitoring to mitigate hemorrhagic and thrombotic complications (Hereditary Hemorrhagic Telangiectasia: Diagnosis and Management, 2024).
Caveats and uncertainty
High-quality randomized controlled trials on anesthetic management in HHT are lacking, and most evidence derives from case reports, small cohorts, and expert consensus. The heterogeneity of vascular lesions and clinical severity in HHT patients limits generalizability. Additionally, the risk of AVMs in critical sites such as the brain or spinal cord may contraindicate certain regional anesthesia techniques, but imaging to exclude these lesions is not always available preoperatively.
The optimal anesthetic agents and monitoring modalities remain undefined, and the balance between bleeding risk and thrombotic complications requires further study. Furthermore, the impact of newer interventional therapies for HHT on anesthetic management has not been systematically evaluated.
How this may change practice
Anesthesiologists should recognize HHT as a high-risk condition for perioperative bleeding and tailor management accordingly. Preoperative consultation with hematology and otolaryngology is advisable to assess bleeding severity and optimize nasal mucosa status. Imaging to exclude AVMs in neuraxial anesthesia target areas should be considered before regional techniques.
Gentle airway manipulation with atraumatic devices and avoidance of nasal instrumentation when possible can reduce epistaxis risk. Invasive hemodynamic monitoring may be warranted for major procedures or patients with significant AVMs. Maintaining stable blood pressure and avoiding hypertension spikes are critical to prevent rupture of fragile vessels.
Multidisciplinary perioperative planning and individualized anesthetic protocols, as illustrated in recent case reports and cohort studies, may improve safety and outcomes. Awareness of the limited evidence base should prompt cautious interpretation and encourage reporting of anesthetic experiences in HHT to build a more robust knowledge base.